Vasculitis

Question 1

What?

Answer 1

Inflammation of blood vessels

Question 2

What can this lead to?

Answer 2

Inflammation/ Ischaemia/ Necrosis of tissues that vessel supplies

Question 3

Primary vasculitis?

Answer 3

Results from an inflammatory response that targets the vessel walls and has no known cause.

Question 4

Secondary vasculitis?

Answer 4

May be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder (e.g. RA or lupus) or cancer

Question 5

3 main categories?

Answer 5

Large, medium and small vessel

Question 6

What is large vessel vasculitis?

Answer 6

Causes granulomatous inflammation predominantly of aorta and its major branches

Question 7

2 main categories of large vessel vasculitis?

Answer 7

Temporal (giant cell) arteritis
Takayasu arteritis (TA)

Question 8

Age of onset in large vessel vasculitis?

Answer 8

GCA - after 50

TA - before 50

Question 9

Who gets takayasu arteritis?

Answer 9

> In East Asian countries
Young women in 2nd and 3rd decades
Females

Question 10

Pathology of large vessel vasculitis?

Answer 10

Granulomatous infiltration of walls of large vessels

Question 11

Takayasu arteritis?

Answer 11

Claudication
Pain in arm muscles e.g. when doing housework
Bruit – carotid artery
BP difference – between left and right side extremities -> pulseless disease
Angiogram

Question 12

GCA?

Answer 12

Temporal arteritis
Prominent temporal arteries
Strong assoc. with polymyalgia rheumatica
Pain in jaw when chewing food
Tongue claudication - linguine artery

Question 13

Features of temporal arteritis?

Answer 13

Unilateral acute temporal headache
Scalp tenderness
Temporary visual disturbances
Blindness
Jaw claudication

Question 14

Clinical features of large cell vascultiis?

Answer 14

Low- grade fever
Malaise
Night sweats
Weight loss
arthralgia
Fatigue
Claudicant symptoms (both upper and lower limbs)
Untreated -> vascular stenosis and aneurysms (reduced pulses and bruits)

Question 15

Investigations for large vessel vasculitis?

Answer 15

ESR, CRP and plasma viscosity elevated
MR angiography –> thickened vessels & vessel stenosis
PET CT – if inflammation present

Question 16

Investigations for GCA?

Answer 16

TEMPORAL ARTERY BIOPSY
- Skip lesions occur so biopsy may be false neg
Ultrasound
- 24- 48 hrs after start steroids

Question 17

Treatment for large vessel vasculitis?

Answer 17

Corticosteroids (prednisolone 40- 60mg)

Methotrexate and azathioprine (may be added)

Question 18

Immunosuppressives in TA?

Answer 18

Leflunamide, methotrexate

Question 19

Immunosuppressives in relapsing or resistant GCA?

Answer 19

Tocilizumab – IL-6 blocking agent

Question 20

How long is GCA treatment?

Answer 20

18 months to 2 years

Question 21

4 types of ANCA associated vasculitis?

Answer 21

Granulomatosis with polyangitis
Microscopic polyangitis
Renal limited vasculitis
Eosinophilic granulomatosis with polyangitis

Question 22

Who gets granulomatosis with polyangitis (GPA/ wagerers)?

Answer 22

Northern Europe
Males >
35- 55 years

Question 23

Symptoms of GPA?

Answer 23

Constitutional symptoms and arthralgia
ENT symptoms
Respiratory symptoms 
Cutaneous
Renal

Question 24

ENT symptoms of GPA?

Answer 24

Nose bleeds 
Deafness 
Recurrent sinusitis 
Nasal crusting (over time can be collapse of nose) 
Epistaxis 
Mouth ulcers 
“Saddle nose” 
Sub- glottic inflammation (trouble breathing, inflammation) 
Proptosis

Question 25

Resp symptoms of GPA?

Answer 25

``` Haemoptysis Cavitating lesions on x-ray Cough Pulmonary infiltrates Diffuse alveolar heamorrhages ```

Question 26

Cutaneous symptoms of GPA?

Answer 26

Palpable purpura | Cutaneous ulcers

Question 27

Renal symptoms of GPA?

Answer 27

Necrotizing glomerulonephritis

Question 28

What is GPA associated with?

Answer 28

cANCA and PR3

Question 29

EGPA?

Answer 29

Characterized by late onset asthma, rhinitis and raised peripheral blood eosinophil count Mono neuritis multiplex

Question 30

Complications of microscopic polyangitis?

Answer 30

Glomerulonephritis

Question 31

Investigations for ANCA associated vasculitis?

Answer 31

ESR, PV and CRP raised Anaemic of chronic disease common U + E (renal involvement?) Anto- neutrophil cytoplasmic antibody (ANCA) Urinalysis (looking for renal vasculitis) CXR Biopsy (of affected area)

Question 32

Management of ANCA assoc vasculitis?

Answer 32

IV steroids and cyclophosphamide

Question 33

Henoch Schonlein purpura?

Answer 33

Acute immunoglobulin A (IgA) mediated disorder Generalized vasculitis involving small vessels of skin, GI tract, kidneys, joints & rarely lungs and CNS Group A streptococcus (most common)

Question 34

Who gets Henoch Schonlein purpura?

Answer 34

Children (2-11) History of upper resp tract infection (few weeks) Rare in infants

Question 35

Symptoms of Henoch Schonlein purpura?

Answer 35

``` Purpuric rash over buttocks and lower limbs Abdominal pain (colicky) Vomiting Joint pain +/- swelling Bloody diarrhea Renal involvement ```

Question 36

Management of Henoch Schonlein purpura?

Answer 36

Self- limiting – settles over weeks to months

Question 37

Investigations for vasculitis?

Answer 37

Urine dipstick FBC, Liver and renal profile, inflammatory markers ANCA and specific antibodies, connective tissue disease screen, compliment levels Imaging investigations – Chest x-ray and/or CT scan Nerve conduction tests (neurological symptoms?) Tissue biopsy (most definitive test) - GOLD

Question 38

ANCA?

Answer 38

Anti- neutrophil cytoplasmic antibodies | Autoantibodies against antigens in cytoplasms of neutrophil granulocytes

Question 39

ANCA in GPA?

Answer 39

cANCA

Question 40

ANCA in EGPA?

Answer 40

pANCA

Question 41

ANCA in MPA?

Answer 41

pANCA